Being the spouse of someone with BCCNS (or any other condition for that matter) can be a challenge. I do not mean to imply for a single second that I regret having Christen as my wife. I don’t. She was chosen by God for me, and I am grateful. She is the best decision I ever made. What I mean is that her having BCCNS spotlights my own shortcomings. I freely admit that I am terrible at recognizing that sometimes, she just wants me to listen. To trulylisten. And to only listen. It is at those times that I realize my real role is to sometimes just support her. Our journey over the past four years has taught me that.
That lesson was crystallized for me on three occasions. The first was when Christen came home from the dermatologist with the news that she no longer suffered from “various unrelated symptoms”, but that there was indeed now a name for it. The second was when she returned from the BCCNS Life Support Network colloquium in St. Louis after having met so many others who shared her plight. And the third was when she wrestled with the notion of whether or not we should have children. It is one thing to bear your own cross, but it’s another thing altogether to cast those same burdens onto those you love.
We researched our options and were delighted to discover that, thanks to recent advances in modern medicine through in vitro fertilization, doctors could safely screen our embryos and identify the genetic marker which indicated a positive finding of BCCNS. Only embryos that were negative for BCCNS would be implanted and we were “guaranteed” a child free from the syndrome.
But, the best laid schemes of mice and men often go awry.
For those of you who don’t have the luxury of knowing my wife, the best one-word description I can give you is “prepared”. No matter what life throws at her, she is fully capable of knocking it out of the park because, somehow for her, life has a way of tipping its pitches — to borrow a baseball metaphor. There simply are no surprises. But, not this time. She was unsettled. Oh, the cribs were assembled. The nursery was done. A year’s supply of matching outfits were hung neatly in the closet. “What to Expect When You’re Expecting” had been read. She was physically ready, but I don’t think she was mentally ready. Not quite yet. And looking back on it, I think I now know why. Everything had been too perfect up to that point – and she was sensing the curve ball.
I was blissfully ignorant.
Christen’s water broke, ironically enough, at 4:00 a.m. on Mother’s Day, May 11, 2008, some twelve weeks prematurely. It is hard to put into words the emotions we felt that morning as we drove the sixty miles to the hospital. “Shock” doesn’t do it justice. “Terror” is probably closer to being the appropriate adjective. She had been to the doctor only five days earlier and everything was normal. We were having twin boys and I think I can speak for Chirsten when I say that we were, in our own ways that morning, processing what was happening and silently praying for their safety.
I recall looking at her that morning as we pulled into the hospital. She had never looked more beautiful to me.
Christen and I lived six hours from family, or as we like to say, “Just fifteen minutes by telephone.” And while it is somewhat ironic that the boys were born on Mother’s Day, the practical effect of that irony is the rest of our families already had separate plans. So, we told them all to enjoy the day together, that the hospital had given her medication to delay delivery, and we spent the day (between contractions) watching the final round of the TPC Championship from a hospital bed – alone together.
Her OBGYN finally came to see us at almost 8:00 p.m. After taking one look at Christen, he told the nurses, “She’s going into labor. Dad, put on these scrubs and meet us at the Delivery Room.”
Do what? No, you said we weren’t going to have them for a few days. You said everything was going to be okay. We told our families to stay away. My mother, and mother-in-law alike, are going to skin me alive for making them miss this moment.
Damn. Curve ball.
The next half hour is a blur for me. It was for Christen as well, but at least she had the luxury of being medicated. William Robert weighed in at 3 lbs, 6 oz and Patrick Reagan was 2 lbs, 12 oz.
They were the second and third most beautiful things in the delivery room at that moment.
Over the next several days, we were swarmed with visits from friends and family alike. But Christen sensed that something was wrong with William. His head was entirely too large for his little body. Macrocephaly is a symptom of BCCNS. And so is hydrocephalus. William had both. You can imagine the combination of excitement and dread that we both shared.
Damn. Curve ball.
For the next three months, we made the commute daily (sometimes twice) to and from the hospital to see them. We were able to have our sons all to ourselves, completely untethered from any nurses or doctors, for six joy-filled hours on Father’s Day.
At some point, a blood sample was drawn from William and sent to a different lab who confirmed that he did, indeed, have BCCNS.
Damn. Curve ball.
Suddenly the doctor who had screened the embryos and his lab were no longer accessible. Phone calls and e-mails went unanswered. But there was no time for search parties. It was becoming clear that William’s head was developing abnormally. Doctors confirmed that he had sagittal craniosynostosis. A suture at the top of his skill had fused together prematurely and was causing his little head to become more and more elongated with each passing day.
Damn. Curve ball.
Meanwhile, Patrick was meeting one developmental milestone after another. For him, it almost seemed too easy. He was our “Champ” – a moniker that he still carries. Our hearts were torn. We had one child who was thriving, almost without any effort whatsoever, and one who never stopped crying because the intracranial pressure build-up meant he had a headache every second of every day. And we were scheduling one doctor’s appointment after the next. Thank God for family and friends.
An incredible team of surgeons at Children’s Medical Center of Dallas removed a 17 cm strip of bone from the top of William’s skull once he got to five pounds and was “big enough” to withstand the anesthesia. For months, Christen or I flew with William to Dallas to be seen by his doctors and to make certain his little helmet (adorned with the requisite Texas Aggies stickers) fit properly. At some point, the travels became too much to bear, so our family was separated while she lived with her parents more closely to the hospital. My only connection with my wife and family came via pictures that were e-mailed to me and phone calls where I tried to memorize the sound of their voices.
After William’s suture healed, he was ready for his VP shunt which would relieve the pressure and ease his crying. The moment he woke up from that surgery, he was an entirely different baby. No longer did his plaintiff whaling reverberate throughout our home. He was immediately transformed into the most loving child imaginable.
Our family circle was complete.
Follow-up visits with the doctors came with a litany of restrictions. He will never be able to play football. Or box. He is supposed to avoid bounce houses or any other activity where he could strike his head. Caps and sunscreen are as much a part of his daily attire as underwear, but for him it’s normal. So what?
I went from being the spouse of someone with BCCNS to also being the father of someone with it. Again, so what? I am the most blessed husband in the world. I watched the woman I love (in the midst of dealing with insufferable guilt – though you will never get her to openly admit it) plan, organize and host a 5K race in our home town that raised thousands of dollars for the BCCNS Life Support Network as a way of giving back to those who had helped us when we needed it most.
If you are considering whether or not you should have children due to the increased risk of transmitting BCCNS, we would only recommend that you pray on it. God gives each of us what we can handle. We support one another with whatever burdens they may bear. That’s what being a family is about.
A curve ball is no different than any other pitch. If it comes across your plate, trust in yourself and swing for the fences. Christen has taught me that. Blissful ignorance only gets you so far.